Figure 2.11 A: Chronic bullous disease of childhood (Courtesy of Dr. Michelle B. Bain) B: Hailey–Hailey disease (Courtesy of Dr. Paul Getz) C: ILVEN (Courtesy of Dr. Paul Getz)
Blistering disorder with onset typically before age 5
Target antigen: 97 kDa Ag (LAD-1 or LABD97): cleaved ectodomain of BPAG2
Presents with annular and herpetiform bullae favoring extensor surfaces/ groin (“crown of jewels” configuration)
Histology: subepidermal bullae with neutrophils in dermal papillae (similar to dermatitis herpetiformis)
